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Favourable and sustained response to anakinra in tumour necrosis factor receptor-associated periodic syndrome (TRAPS) with or without AA amyloidosis
  1. Laura Obici1,
  2. Antonella Meini2,
  3. Marco Cattalini2,
  4. Silvana Chicca3,
  5. Marco Galliani3,
  6. Simona Donadei1,
  7. Alessandro Plebani2,
  8. Giampaolo Merlini1
  1. 1Amyloidosis Research and Treatment Center, Biotechnology Research Laboratories and Department of Biochemistry, Fondazione IRCCS Policlinico San Matteo and University of Pavia, Pavia, Italy
  2. 2Immuno-Rheumatology Unit, Clinica Pediatrica, University of Brescia, Brescia, Italy
  3. 3Nephrology Unit, Ospedale Sandro Pertini, Roma, Italy
  1. Correspondence to Professor Giampaolo Merlini, Amyloid Research and Treatment Center, Biotechnology Research Laboratories, Fondazione IRCCS Policlinico San Matteo Viale Golgi, 19, 27100 Pavia, Italy; gmerlini{at}unipv.it

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Tumour necrosis factor receptor-associated periodic syndrome (TRAPS) is a dominantly inherited autoinflammatory disease characterised by recurrent episodes of fever, arthritis and serositis. In adulthood, inflammation often becomes persistent1 and AA amyloidosis may occur. Evidence of dysregulated secretion of proinflammatory cytokines, particularly interleukin 1 (IL-1) beta, in autoinflammatory diseases recently supported anakinra, the recombinant IL-1 receptor antagonist, as a target therapy for most of these conditions.2 3 In TRAPS, response to anakinra was reported in two case reports and in one prospective trial, in which five patients were treated for a mean duration of 11.4 months.4,,6 However, longer follow-up and additional observations are warranted. We report the long-term efficacy and safety of anakinra in seven TRAPS patients with and without …

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